�A u s t r A l i A n & n e w Z e A l A n d J o u r n A l o f P h l e b o l o g y V o l u m e 1 0 ( 1 ) : J A n u A r y 2 0 0 7 �A u s t r A l i A n & n e w Z e A l A n d J o u r n A l o f P h l e b o l o g y V o l u m e 1 0 ( 1 ) : J A n u A r y 2 0 0 7

p-p � -

Introduction

Patients with venous disease often exhibit dermatological

changes. Sometimes these skin changes are the only

clue to an appropriate list of differential diagnoses. Venous

insufficiency is the most common venous disease which

presents with a range of skin changes. Most people are

familiar with venous eczema, lipodermatosclerosis and

venous ulcers as manifestations of long-term venous

insufficiency. The less familiar are changes such as

acroangiodermatitis and pigmented purpuric dermatoses.

Dilatation of venous structures is not always due to

venous incompetence and could be primary or secondary

to systemic disease, or environmental influences such as

actinic damage and radiation. This paper will discuss the

dermatological manifestations of venous insufficiency as

well as other forms of vascular ectasias that may present

in a similar fashion to venous incompetence. The second

instalment will focus on dermatological manifestations

of haematologic disease and in particular thrombo-

inflammatory conditions. The third instalment will discuss

vascular anomalies.

Skin Manifestations Of Venous Insufficiency

Common Manifestations

Common manifestations of venous insufficiency include

oedema, corona phlebectasia paraplantaris (Figure

1), stasis dermatitis, pigmentory changes (hyper- and

hypo-), atrophie blanche, lipodermatosclerosis, and skin

ulceration. Less common manifestations include pigmented

purpuric dermatoses, and acroangiodermatitis. Superficial

thrombophlebitis (STP) can also occur in association with

venous incompetence but will be discussed in the second

instalment of this paper (Figure 2).

Venous hypertension is responsible for many

manifestations of chronic venous insufficiency (CVI)

including oedema, red cell extravasation, perivascular

fibrin deposition, impaired arterial inflow, and other

locally mediated disturbances. The local tissue sequelae of

CVI are due to impaired clearance of cellular metabolites

secondary to concurrent damage to the lymphatic system.

One of the earliest manifestations of venous hypertension

is lower limb oedema. Oedema is defined as a clinically

apparent increase in the interstitial fluid volume. Oedema

that retains an indentation when pressed with a finger is

called pitting oedema. Systemic causes of oedema include

congestive cardiac failure, hepatic insufficiency, renal

failure, pretibial oedema in myxoedema, hypoproteinemia,

and other systemic disorders. Lymphoedema may be a sign

of primary lymphatic outflow obstruction, or it may be

secondary to the overproduction of lymph due to severe

Dermatological

manifestations of Venous

Disease: Part i

Kurosh Parsi,Departments of Dermatology, St. Vincent’s Hospital and Sydney Children’s HospitalSydney Skin & Vein Clinic, Bondi Junction, NSW, Australia

A R T I C L E

Review

Address Correspondence to: Kurosh Parsi, Sydney Skin and Vein Clinic, Suite 2102, Westfield Tower 1, 520 Oxford Street, Bondi Junction NSW 2022, Australia Telephone: +612 9386 0211 Facsimile: +612 9386 0258 Email: kparsi@ozemail.com.au

Skin changes are one of the earliest signs of venous hypertension. Some of these changes such as venous eczema are common and easily identified whereas other changes such as acroangiodrmatitis are less common and more difficult to diagnose. Other vein related and vascular disorders can also present with specific skin signs. Correct identification of these skin changes can aid in making the right diagnosis and an appropriate plan of management. Given the significant overlap between phlebology and dermatology, it is essential for phlebologists to be familiar with skin manifestations of venous disease. This paper is the first installment in a series of 3 and discusses the dermatological manifestations of venous insufficiency as well as other forms of vascular ectasias that may present in a similar fashion to venous incompetence.

abstract

� V o l u m e 1 0 ( 1 ) : J A n u A r y 2 0 0 7 A u s t r A l i A n & n e w Z e A l A n d J o u r n A l o f P h l e b o l o g y

K.Parsi

venous hypertension (the so-called phlebolymphoedema).

Usually, lower leg oedema is symmetrical in patients with

systemic disorders.

Oedema of venous insufficiency is quite often associated

with skin changes. Venous eczema (Figure 3) is usually the

earliest cutaneous manifestation of venous insufficiency,

and it may be a precursor to hyperpigmentation and

lipodermatosclerosis. The medial ankle is most frequently

involved revealing erythematous, scaling, and sometimes

exudative, weeping patches and plaques (Figure 4).

Secondary bacterial infection with staphylococcus aureus

can cause typical crusting. Pseudomonal colonisation

is a common complication. In long-standing cases,

lichenification may occur as a consequence of chronic

scratching and rubbing.

The development of contact dermatitis is especially

problematic in the treatment of patients with venous

eczema. Topical treatments, including neomycin,

bacitracin and some topical corticosteroids, have been

reported to cause contact sensitization in patients with

venous eczema.1 Contact dermatitis should be considered

when venous eczema becomes clinically worse or does

not improve despite appropriate topical treatment. Patch

testing will be indicated. Topical antibiotics such as

neomycin and popular preparations such as Kenocomb

should never be used on open venous ulcers.

Venous hypertension leads to red cell extravasation.

Breakdown of red cells leads to haemosiderin

deposition and cutaneous pigmentation (Figure 5).

Hyperpigmentation secondary to red cell extravasation

is difficult to treat and not responsive to hydroquinone

or other standard bleaching agents used to treat

hypermelanosis. Treatment of the underlying venous

hypertension can help in lightening the pigmentation but

in general once haemosiderin pigmentation is present, it is

mostly irreversible. Post-inflammatory hypopigmentation

may occur which can even result in depigmentation. This

should be differentiated from atrophie blanche.

Atrophie blanche is a specific type of dermal scarring

usually presenting as a small reticulated porcelain white

patch. Dermoscopic examination of atrophie blanche

demonstrates dilated capillary loops interspersed within

the dermal scars. Atrophie blanche is not specific

for venous hypertension and can be a manifestation

of livedoid vasculopathy as discussed below. Atrophie

blanche is caused by obstruction of dermal arterioles and

infarction of the skin supplied by these vessels (Figures

6-8). This obstruction can be secondary to venous

hypertension or thrombo-occlusive vasculitis involving

dermal arterioles. Skin in this condition is avascular

and prone to ulceration and necrosis. Vasculitis can

also lead to identical white scars of atrophie blanche

on the legs. Other systemic diseases, such as systemic

lupus erythematosis (SLE), rheumatoid arthritis, and

Klinefelter syndrome can result in skin ulcerations that

heal with atrophie blanche-like lesions.2

Lipodermatosclerosis (LDS) is the induration of skin and

the underlying fat in the lower legs (Figure 9). It usually

extends from the ankles to the lower border of the calf

muscle. Histologically, there is a septal panniculitis and

sclerosis of the overlying skin.2 A less-known form is the

so called ‘acute lipodermatosclerosis’ which is primarily

an acute panniculitis. Acute lipodermatosclerosis can

be confused with acute cellulitis or even acute venous

eczema. Eventual induration of skin and the underlying

adipose tissue leads to the more familiar clinical picture

of chronic lipodermatosclerosis. Progression of disease

leads to the classic inverted champagne bottle appearance.

The induration and lack of perfusion of the tissue makes

it prone to ulceration.

Most chronic lower leg ulcers have a venous aetiology. 3 Most venous ulcers are caused by superficial venous

incompetence. 4 Only a minority are caused by deep vein

incompetence or obstruction. Non-healing ulcers of the

medial ankle are most likely due to underlying venous

hypertension. These areas are especially prone to venous

hypertension because their drainage largely depends on

the competence of the great saphenous system and its

associated perforating veins. The incompetent Cockett

perforators along the posterior arch tributary of the great

saphenous vein (GSV) play a crucial role in the altered

haemodynamics of this area.

Venous ulcers are usually larger but shallower than other

ulcers, have a moist granulating base and an irregular

border (Figure 10). Calcification is a common finding.

The tissue surrounding these ulcers may exhibit signs

of stasis dermatitis and other changes such as atrophie

blanche and hyperpigmentation.

Non-healing, nodular areas of venous ulcers should be

biopsied to exclude neoplastic change. The most common

tumour is squamous cell carcinoma, however rarely basal

�A u s t r A l i A n & n e w Z e A l A n d J o u r n A l o f P h l e b o l o g y V o l u m e 1 0 ( 1 ) : J A n u A r y 2 0 0 7

Dermatological Manifestations of Venous Disease: Part 1

cell carcinomas may develop too. 3 Malignant melanoma

may present as a lower leg ulcer. Other important

differentials include arterial, neuropathic, traumatic,

pyoderma gangrenosum, vasculitis, infection, and self-

induced artefact. Biopsies should be taken from the edge

of the ulcer and not from its necrotic base. Multiple

biopsies may be required for the diagnosis. Care must be

taken in providing haemostasis. It is usually not possible

to close the biopsy site with suture as the tissue is friable.

Steri-strips can be applied but compression should be

used to prevent bleeding from the biopsy site.

Livedoid Vasculopathy

Atrophie blanche is not always a manifestation of CVI but

can be a sign of livedoid vasculopathy (LV). This condition

is characterized by ulceration associated with a segmental

pattern of reticulate purpura and atrophie blanche affecting

the lower limbs (Figure 11). It has been falsely referred to as

livedoid vasculitis, although true vasculitis is not a feature

of this condition. It has also been described as ‘livedo

reticularis with summer ulcerations’. LV is a thrombo-

occlusive process. Histologically, it shows fibrin deposition

within both the wall and the lumen of affected vessels but

there is no evidence of a true vasculitis. 5

LV has been associated with Raynaud’s phenomenon

and acrocyanosis. Patients with LV may have a history

of recurrent leg ulcerations. Such patients may have

thrombophilic abnormalities such as factor V Leiden

mutation, protein C deficiency, hyperhomocysteinemia,

abnormalities in fibrinolysis, and increased platelet

activation. 6

Pseudo-Kaposi’s Sarcoma (Acroangiodermatitis)

Another unique feature of CVI is the development

of violaceous plaques and nodules on the legs and

dorsal aspects of the feet (Figures 12-13). These lesions

frequently undergo painful ulceration and can be

clinically indistinguishable from classic Kaposi’s Sarcoma

(KS). This clinical appearance has led this entity to be

Figure 5: Typical venous hyper- pigmentation of the medial gaiter area.

Figure 6: Porcelain white scars of atrophie blanche in an area of lipoderma-tosclerosis.

Figure 7: Diagram demonstrat-ing the arteriolar supply of the skin corresponding with cutaneous infarcts of atrophie blanche.

Figure 8: Close-up image of atrophie blanche, interspersed punctate telangiectasias and underlying varicose veins.

Figure 1: Corona phlebectatica para-plantaris.

Figure 2: Superficial Thrombophlebitis (STP) of a Great Saphenous vein tributary.

Figure 3: Venous eczema over the dorsum of the foot. Venous hyperpigmentation over the lateral aspect of the foot.

Figure 4: Discoid eczema associated with venous hypertension.

10 V o l u m e 1 0 ( 1 ) : J A n u A r y 2 0 0 7 A u s t r A l i A n & n e w Z e A l A n d J o u r n A l o f P h l e b o l o g y

K.Parsi

called pseudo–KS or acroangiodermatitis. Pseudo-KS

is a hyperplasia of pre-existing vasculature. In contrast,

KS is a vascular tumour associated with Human Herpes

Virus type 8 (HHV 8). Vascular proliferation in KS is

independent of the existing vessels.

Pseudo-KS is usually seen as a complication of

severe CVI. Less commonly, congenital or acquired

arteriovenous malformations, and arteriovenous fistulas

can result in localised venous hypertension and the

development of pseudo–KS. Pseudo-KS can also

occur distal to the site of arterio-venous fistulas for

haemodialysis. These lesions may resolve after closure,

thrombosis or surgical elimination of the shunt. Pseudo-

KS has also been described in association with hepatitis

C and in some vascular malformations such as Klippel-

Trenaunay syndrome (KTS). Bilateral lesions are usually

associated with CVI, whereas unilateral lesions suggest an

underlying arterio-venous malformations or a high flow

lesion. Patients occasionally experience pruritus and pain.

Ulceration and bleeding may also occur.

Pigmented Purpuric Dermatoses

Pigmented purpuric dermatoses (PPD) are a group of

conditions characterized by extravasation of red cells

and marked haemosiderin pigmentation of the skin. This

condition has been wrongly called ‘capillaritis’, although

there is no evidence of a true vasculitis. The hallmark

of PPD is its characteristic orange-brown, speckled,

cayenne pepper–like discoloration. The aetiology of PPD

is currently under investigation. Venous hypertension,

contact dermatitis, and certain drugs have been implicated

in the pathogenesis of PPD. Other associations are

hypersensitivity to food dyes and preservatives such as

tartrazine, and clothing dye dermatitis (especially khaki

dyes). Drugs such as aspirin, glipizide, thiamine and

interferon-alfa have been implicated. 7-10 The author’s

recent research has discovered coagulation abnormalities

and in particular platelet function abnormalities in

this group of patients (in press). Differential diagnoses

include angiocentric mycosis fungoides, venous

hyperpigmentation, leukocytoclastic vasculitis, petechial

Figure 9: Lipodermatosclerosis associated with atrophie blanche.

Figure 10: Advanced venous ulcers causing fixed foot deformity. This patient received UGS and all ulcers healed. However, she eventually required a left below knee amputation to facilitate mobility.

Figure 11: Livedoid vasculopathy secondary to protein S deficiency.

Figure 12: Acroangio- dermatitis secondary to venous hypertension can resemble Kaposi’s sarcoma.

Figure 13: Hyperpigmentation secondary to acroangiodermatitis can resemble venous hyperpigmentation.

Figure 14: Schamberg’s Disease (taken from Schamberg.J.F, A peculiar progressive pigmentary disease of the skin, British Journal of Dermatology, 1901, 13:1-5).

Figure 15: Itchy purpura.

11A u s t r A l i A n & n e w Z e A l A n d J o u r n A l o f P h l e b o l o g y V o l u m e 1 0 ( 1 ) : J A n u A r y 2 0 0 7

Dermatological Manifestations of Venous Disease: Part 1

haemorrhage and scurvy.

A number of clinical patterns are recognized all

demonstrating a similar histologic appearance. These

include Schamberg’s disease (or progressive pigmented

purpuric dermatosis) (Figure 14), itchy purpura of

Lowenthal (Figure 15), lichen aureus (Figures 16-17),

purpura annularis telangiectodes (Majocchi disease)

(Figure 18), pigmented purpuric lichenoid dermatosis

of Gougerot and Blum (Figures 19), unilateral PPD

(Figures 20-21) and eczematoid-like purpura of Doucas

and Kapetanakis. A granulomatous variant of PPD has

also been reported.

These subclass all share common histopathologic features

of red cell extravasation, haemosiderin deposition and a

lymphocytic infiltrate. A lichenoid inflammatory infiltrate

is present in the Gougerot-Blum sub-type. Spongiosis is

present in the Doucas and Kapetankis sub-type. Older

lesions tend to be less inflamed and extravasated red cells

may no longer be present. In the author’s experience,

these sub-classifications are somewhat artificial and one

patient may exhibit multiple sub-types. Lichen aureus

is commonly found in association with venous disease

and sometimes directly over an underlying incompetent

perforator. Treatment of the underlying localised venous

hypertension can help in the resolution of lichen aureus

(Figures 16-17). Topical steroids are possibly helpful only

if pruritus is present but otherwise have a limited role.

The underlying condition and in particular coagulation

abnormalities should be investigated and excluded. Patch

testing, other forms of allergy testing and food elimination

diets may be appropriate. Aspirin, vitamin E, fish oil and

other forms of drugs and supplements interfering with

platelet function should be stopped, if appropriate.

Cutaneous Vascular Anomalies

Cutaneous vascular anomalies may be classified into

the following categories of tumours, malformations

and ectasias. Tumours may be congenital or acquired,

benign or malignant and hyperplastic or neoplastic. The

Figure 16a: Lichen aureus before treatment.

Figure 16b: Lichen aureus after treatment.

Figure 17b: Lichen aureus after treatment.

Figure 18: Majocchi disease.Figure 17a: Lichen aureus before treatment.

Figure 19: Pigmented purpuric lichenoid dermatosis of Gougerot and Blum.

Figure 20: Unilateral PPD. Figure 23: Telangiectasias in association with underlying varicose veins.

Figure 22: Venous lake on the lower lip.

Figure 21: Unilateral PPD.

12 V o l u m e 1 0 ( 1 ) : J A n u A r y 2 0 0 7 A u s t r A l i A n & n e w Z e A l A n d J o u r n A l o f P h l e b o l o g y

K.Parsi

most common tumour of childhood is haemangioma.

Haemangiomas are not present at birth and typically

undergo involution. Malformations are errors of

morphogenesis and are present at birth. They do not

spontaneously involute. Vascular ectasias are dilatations

of preexisting normal vessels. This paper will discuss

vascular ectasias not necessarily associated with venous

incompetence. The third installment of this paper will

discuss vascular tumours and malformations.

Vascular Ectasias

Dilatation of blood vessels can be due to increased

intravascular pressure or loss of elasticity of the vessel

wall. Varicose veins are dilated vessels demonstrating

increased muscularity and loss of elastic fiber. Vascular

ectasia could also be due to actinic damage resulting in

elastolysis of the vessel wall.

VenousLake

Venous lakes are probably the most common vascular

ectasias. These are dark blue phlebectasias that tend

to occur on sun-exposed skin, especially on the lower

lips or ears of elderly patients (Figure 22). Chronic

actinic damage injures the vascular adventitia and the

dermal elastic tissue, causing dilatation of normal venous

structures. Treatment with sclerotherapy for larger

lesions and vascular laser therapy for smaller lesions is

usually successful. Malignant melanoma is a differential

diagnosis and should be excluded. Venous lakes, as

against melanomas, are compressible and empty with

compression. Venous lakes are benign lesions but can

bleed or thrombose causing pain.

Angiokeratomas

Angiokeratomas are a group of vascular ectasias that

involve the papillary dermis. Angiokeratomas are not

true angiomas but rather ectasias of pre-existing vessels.

Overall, altered haemodynamics typically caused by

trauma appear to produce telangiectatic vessels of the

papillary dermis with an overlying reactive hyperkeratosis

to the epidermis. These lesions may also produce

papillomatosis, and acanthosis of the epidermis. 11

angiokeratoma of fordyce. These are typically

asymptomatic, 2-5 mm, blue-red papules with a scaly

surface located on the scrotum, shaft of penis, labia

majora, inner thigh, or lower abdomen. They are dilated

ectatic capillaries in the superficial dermis with overlying

epidermal hyperplasia. These lesions bleed easily.

Angiokeratomas occur in the presence of varicoceles

or vulvar varices and venous hypertension has been

implicated in its pathogenesis. 12 If treatment is needed,

then locally destructive methods have been used. Light

hyfrecation may not provide adequate haemostasis and

bleeding may occur. Vascular laser photocoagulation

usually provides adequate treatment.

angiokeratoma circumscriptum (ac). This is a rare type

of angiokeratoma with a unilateral distribution of discrete

papules and nodules localized to a small area of the leg or

trunk. Due to its unilateral and truncal distribution, AC

has also been called angiokeratoma corporis neviform.

AC has been classified as a capillary malformation. It

has been reported to coexist with angiokeratoma of

Fordyce and caviar spots (angiokeratomas of the tongue).

It can be found in association with other vascular

malformations and haemangiomas. Recurrent bleeding

can occur. Women are affected more commonly than

men, in a ratio of approximately 3 to 1. 13 These lesions

respond well to vascular laser treatment.

Figure 24: Facial telangiectasias. Figure 25: Post-radiation telangiectasias.

Figure 26: Mat telangiectasia of scleroderma.

13A u s t r A l i A n & n e w Z e A l A n d J o u r n A l o f P h l e b o l o g y V o l u m e 1 0 ( 1 ) : J A n u A r y 2 0 0 7

Dermatological Manifestations of Venous Disease: Part 1

angiokeratoma corporis Diffusum (fabry’s disease).

This is an X-linked disorder caused by a deficiency

of the lysosomal enzyme alpha-galactosidase. This

inborn error of metabolism results in deposition of

neutral glycosphingolipids in the lysosomes of most cells.

The most affected cell is the vascular endothelium. 14

Angiokeratomas form and tend to concentrate between

the umbilicus and the knees but usually spare the head.

Lymphoedema and varicose veins are present. Renal

failure, cardiac failure and cerebrovascular disease are

the major causes of death. Heterozygous females may

develop angiokeratomas but experience a milder clinical

course.

Telangiectasias

Telangiectasias are dilated venules, arterioles or

arteriovenous malformations visible on the skin or

mucosal surfaces. Telangiectasias that develop in the

absence of any preceding or coexisting cutaneous or

systemic disease are considered to be primary or essential.

Telangiectasias resulting from or in association with a

known disease are classified as secondary

leg telangiectasias (spider veins). These vessels are

dilated post-capillary venules demonstrating backflow

from incompetent reticular (feeder) veins or directly

from incompetent tributary or even truncal veins (Figure

23). Doppler examination may demonstrate reflux in

even very small telangiectatic vessels. Telangiectasias

associated with venous incompetence are usually dark

red. Larger bluish vessels are termed venulectasias. Not all

leg telangiectasias are secondary to venous incompetence

and essential telangiectasias are commonly found near

the feet and ankles. Gold standard for treatment of leg

telangiectasias is sclerotherapy. The underlying venous

incompetence must be treated first, before any attempt

is made at sclerosing the telangiectasias. Vascular laser

therapy is unlikely to be effective if underlying venous

incompetence is present.

facial telangiectasias. Telangiectasias seen on the face

and trunk are usually due to actinic damage. Facial

telangiectasias may also be associated with flushing

and vascular instability as seen in rosacea or carcinoid

syndrome (Figure 24). Best treatment for these lesions

is vascular laser therapy. Peri-alar telangiectasias usually

demonstrate higher flow volumes and are more difficult

to treat. They are more common in people suffering with

sinus disease and chronic nasal congestion. This can lead

to recurrent peri-alar telangiectasias despite adequate

treatment. Vascular laser therapy may need to be repeated

a number of times for larger peri-alar telangiectasias or

sclerotherapy can be attempted.

Post-radiation telangiectasias. Radiotherapy can lead

to appearance of telangiectasias up to 20 years after the

procedure. The radiation damage causes destruction of

elastic fibers within vessels and leads to formation of the

telangiectasias (Figure 25). Malignancy and in particular

squamous cell carcinoma can develop in these areas.

mat telangiectasias. These are patches of telangiectasias

seen in patients with scleroderma and CREST syndrome

(Figure 26). They present as flat and non-pulsatile vessels

appearing as discrete mats. This should not be confused

with telangiectatic matting which is a complication of

surgery and sclerotherapy.

telangiectatic matting. This is a form of neovascularisation

which occurs as a complication of sclerotherapy or surgery

(Figure 27). Patches of very small telangiectasias appear

over the sites of recanalized vessels. Inflammation seems

to play a role in its pathogenesis. Inflammatory cytokines

with angiogenic properties probably play an important role

Figure 27a: Telangiectatic matting – pre-sclerotherapy.

Figure 27b: Telangiectatic matting – post-sclerotherapy.

Figure 28: Poikiloderma of Civatte.

14 V o l u m e 1 0 ( 1 ) : J A n u A r y 2 0 0 7 A u s t r A l i A n & n e w Z e A l A n d J o u r n A l o f P h l e b o l o g y

K.Parsi

in the pathogenesis of this condition. Other associations

include concurrent use of female hormonal supplements

such as oral contraceptives and hormone replacement

therapy. There are usually underlying small incompetence

veins in direct association with these vessels.

Poikiloderma of civatte. Poikiloderma of Civatte refers

to a reticulate erythematous pigmentation seen on the

sides of the neck (Figure 28). Areas shaded by the chin are

spared. The term poikiloderma refers to the combination

of atrophy, telangiectasias, and pigmentary changes

(both hypo- and hyper-pigmentation). It occurs most

commonly in fair-skinned middle-aged or elderly females.

Chronic exposure to ultraviolet light appears to be a

primary aetiologic factor but photosensitizing chemicals

in perfumes or cosmetics have also been implicated. 15

Periungual telangiectasias. These are telangiectasias along

the proximal nailfold. They are visualized dermoscopically

as dilated capillary loops. These are pathognomonic signs

of connective tissue diseases, such as lupus, scleroderma,

and dermatomyositis.16

generalised essential telangiectasia (get). This refers

to the syndrome of widespread primary telangiectasias

of unknown aetiology. There is no reported association

with venous disease or actinic damage. Familial cases

have been reported with an autosomal dominant

pattern of inheritance.16, 17 It is termed ‘generalised’

because of the widespread anatomic distribution of the

telangiectasias. However, GET usually starts as light pink

linear or punctuate telangiectasias on the feet, ankles,

and distal legs and may gradually spread (Figures 29-30).

When the lesions become confluent, the skin appears

diffusely erythematous. Involvement of the oral mucosa

and conjunctiva has been reported. GET is usually

asymptomatic, but tingling, burning or numbness is

occasionally reported.18 The age of onset is usually in the

fourth or fifth decade, but it may be observed in younger

adults

hereditary benign telangiectasias. This condition

resembles GET. It commences in early childhood, and is

usually present on sun exposed skin.

hereditary haemorrhagic telangiectasia (hht- osler-

rendu-Weber disease). These lesions are arteriovenous

malformations (AVM) of the microvasculature. They

usually appear as punctuate dark red elevated lesions on

mucous membranes, face and distal limbs. Lesions may

involve the tongue, retina, lung and the brain (Figure 31).

HHT lesions appear during the third or fourth decade

of life. Recurrent epistaxis is usually the presenting

symptom. Coagulation factor deficiency such as Von

Willebrand’s disease may be present. Family screening

may be indicated.

unilateral naevoid telangiectasia (unt). In this

condition, patches of telangiectasia present in a unilateral

linear distribution. The segmental pattern suggests a

somatic mosaicism. Pregnancy, chronic liver disease and

other states of oestrogen excess are implicated in its

pathogenesis. The third and fourth cervical dermatomes

are the most common sites, but the thoracic dermatomes

and scattered distant sites may also be involved.2

telangiectasia macularis eruptiva Perstans (tmeP).

This is the telangiectatic form of cutaneous mastocytosis.

The lesions appear as brown telangiectatic macules that

may be generalized in distribution. When a lesion of other

forms of mastocytosis such as urticaria pigmentosa or

mastocytoma is stroked, it typically urticates, becoming

pruritic, oedematous, and erythematous. This change is

referred to as the Darier sign, which can be explained on

the basis of mast cell degranulation induced by physical

Figure 29: Generalised Essential Telangiectasias (GET).

Figure 30: Generalised Essential Telangiectasias (GET).

Figure 31: Hereditary Haemmorhagic Telangiectasias (HHT).

Figure 32: Spider naevus of the forehead.

15A u s t r A l i A n & n e w Z e A l A n d J o u r n A l o f P h l e b o l o g y V o l u m e 1 0 ( 1 ) : J A n u A r y 2 0 0 7

Dermatological Manifestations of Venous Disease: Part 1

stimulation. The Darier sign is usually negative in TMEP.

This is because the lesions of TMEP are paucicellular

and, therefore, mast cells may not be present in sufficient

numbers for significant degranulation to occur.19 Diagnosis

of TMEP is made on skin biopsy. Anaesthetic should be

injected without adrenalin adjacent to, and not directly

into, the lesion to avoid mast cell degranulation, which

would make histologic examination difficult.

ataxia telangiectasia (at). This is an autosomal

recessive immunodeficiency characterized by progressive

neurologic impairment and cerebellar ataxia. Patients

are susceptible to sinopulmonary infections, impaired

organ maturation, and a predisposition to malignancy.

Oculo-cutaneous telangiectasias have a later onset than

ataxia, first noticed in childhood and sometimes not until

adolescence. Ocular telangiectasias extend from the lateral

canthus in the equatorial region of the conjunctivae

towards the corneal limb. Telangiectasias may also involve

the ears, eyelids, the cubital and popliteal fossae. The

telangiectases are predominantly of venous origin and are

not AV malformations.20

spider naevi. Spider naevi are dilated ascending

arterioles. They usually exhibit radiating thin-walled

vessels in association with a central arteriole (Figure 32).

These lesions are also referred to as spider angiomas and

naevus araneus. Spider naevi are not vascular proliferations

but occur as a result of the dilation of preexisting vessels.

They demonstrate arterial flow on Doppler examination.

Compression of the central arteriole produces blanching.

When released, the lesion quickly refills from the central

arteriole. Spider naevi are acquired lesions present in

10-15% of healthy adults and young children. Lesions

are found most commonly on the face, neck, chest and

arms. In young children, spider naevi are common on

the backs of the hands and fingers. Hormonal changes

play a role in pathogenesis of these lesions. Many women

develop lesions during pregnancy or while taking oral

contraceptives. The lesions usually resolve spontaneously

6 to 9 months after delivery or after discontinuing oral

contraceptives. Numerous lesions are seen in patients with

significant hepatic disease such as advanced hepatitis C.

R e f e r e n c e s

1. Crowe MA. Contact Dermatitis. In. No date [cited 2006 Dec

21] Available from http://www.emedicine.com/ped/topic2569.

htm.

2. Weedon D, Strutton G. Skin Pathology. New York: Churchill

Livingstone; 1997.

3. Mekkes JR, Loots MAM, Van Der Wal AC, Bos JD. Causes,

investigations and treatment of leg ulceration. Br J Dermatol

2003;148:388-401.

4. Simon DA, Dix FP, McCollum CN. Management of venous

ulcers. BMJ 2004;328:1358-1362.

5. Freedberg IM, Eisen AZ, Wolff K, Austen KF, Goldsmith LA,

Katz S. Fitzpatrick’s Dermatology in General Medicine. 6 ed.

New York: McGraw-Hill Medical Publishing Medicine; 2003.

6. Leonard A, Pomeranz MK, Franks AG, Jr. A case of

livedoid vasculopathy in a 22-year-old man. J Drugs Dermatol

2004;3(6):678-679.

7. Dowd PM, Champion RH. Purpura. 6 ed. Oxford: Blackwell

Science; 1998.

8. Newton RC, Raimer SS. Pigmented Purpuric Eruptions.

Dermatol Clin 1985;3:165-169.

9. Taketuchi Y, Chinen T, Ichikawa Y, al. e. Two cases of unilateral

pigmented purpuric dermatosis. J Dermatol 2001;28:493-498.

10. Tristani-Firouzi P, Meadows KP, Vanderhooft S. Pigmented

purpuric eruptions of childhood: a series of cases and review of

literature. Pediatr Dermatol 2001;18(4):299-304.

11. Champion RH, Burton JL, Burns DA, Breathnach SM.

Rook/Wilkinson/Ebling: Textbook of Dermatology. 6 ed. Oxford:

Blackwell Science; 1998. vol 1: 592-595

12. Schaffer JJ, De Leo V. Angiokeratoma of the scrotum. In. No

date [cited 2006 Dec 21] Available from http://www.emedicine.

com/derm/topic28.htm.

13. Champion RH, Burton JL, Burns DA, Breathnach SM.

Rook/Wilkinson/Ebling: Textbook of dermatology. 6 ed. Oxford:

Blackwell Science; 1998. vol 1: 592

14. Spitz JL. Genodermatoses: A Clinical Guide to Genetic Skin

Disorders. 2 ed. New York: Lippincott Williams & Wilkins; 2005.

15. Champion RH, Burton JL, Burns DA, Breathnach SM. Rook/

Wilkinson/Ebling: Textbook of Dermatology. Oxford: Blackwell

Science; 1998. vol 2: 1790

16. Singh VN. Dermatologic manifestations of cardiac disease.

In. No date [cited 2006 Dec 21] Available from http://www.

emedicine.com/derm/topic548.htm.

17. Long D, Marchman G. Generalised essential telangiectasia.

Australas J Dermatol 2004;45:67-69.

18. Green D. Generalized Esstenial Telangiectasia. In. No date

[cited 2006 Dec 21] Available from http://www.emedicine.com/

derm/topic164.htm.

19. Sarkany RPE, Monk BE, Handfield-Jones SE. Telangiectasia

macularis eruptiva perstans: a case report and review of the

literature. Clin Exp Dermatol 1998;23:38-39.

20. Paller AS. Ataxia Telangiectasia. In: Freedberg IM, Eisen AZ,

Wolff K, Austen KF, Goldsmith LA, Katz S, editors. Fitzpatrick’s

Dermatology in General Medicine. 6 ed. New York: McGraw-Hill

Medical Publishing Medicine; 2003. vol 2 p. 1833-1836.